Contributors GP Diller, K Dimopoulos, and SJ Wort designed the study. R Alonso-Gonzalez, R Inuzuka, G Giannakoulas, L Castle, and AE Lammers
collected and analysed data related to BNP and outcome. J Hopper, A Uebing, L Swan, and MA Gatzoulis helped in preparing the
manuscript and analysing the data. GP Diller combined the results, performed the final data analysis, and wrote the manuscript.

Accepted 7 February 2012

Published Online First 7 March 2012

Abstract

Objective To assess the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in patients with Eisenmenger
syndrome.

Design Retrospective study.

Setting Tertiary centre for adult congenital heart disease.

Patients All patients with Eisenmenger syndrome (n=181, age 36.9±12.1 years, 31% with Down syndrome) in whom BNP concentrations were
measured as part of routine clinical care were included.

Main outcome measures The study end point was all cause mortality.

Results During a median follow-up period of 3.3 years, 20 patients (7 with Down syndrome) died. Higher BNP concentrations were predictive
of all cause mortality on univariate analysis in patients with or without Down syndrome. On multivariable Cox proportional
hazard analysis, BNP predicted survival independently of renal function, Down syndrome, or 6 min walk test distance (p=0.004).
Temporal increases in BNP concentration were also found to predict mortality. Treatment with disease targeting therapies was
associated with a significant reduction in BNP concentrations.

Conclusions BNP concentrations predict outcome in contemporary Eisenmenger patients. Increases in BNP concentrations over time are also
of prognostic significance. In addition, disease targeting therapies may help to reduce BNP concentrations in this population,
while treatment-naïve patients have static or rising BNP concentrations.

Footnotes

Competing interests Dr Diller has received unrestricted educational grants from Actelion, UK and has served on the advisory board of Actelion,
Germany. Professor Gatzoulis has served on the advisory board of Actelion, Pfizer, and GlaxoSmithKline and has received unrestricted
educational grants from Actelion and Pfizer, UK. Dr SJ Wort has received educational grants from Actelion and has served on
the advisory boards of Pfizer, Bayer and Eli-Lilly.